Retinoblastoma: Perry’s Story

To meet Perry Zimmerman, you would think you're meeting a typical, albeit cute and precocious, five-year-old. What you wouldn't know was that Perry has faced and conquered more life-threatening obstacles in her short life than most people could even begin to imagine.

The Journey Begins

Perry's father, Larry, had the inherited version of retinoblastoma, which is the most common form of eye cancer in children. Since each child of a parent with inherited retinoblastoma has about a 50 percent chance of being born with the disease, Larry and his wife, Anne, who was pregnant with Perry at the time, opted to have amniocentesis, a common prenatal test of the amniotic fluid, to see if Perry had inherited the geneticmutation responsible for the disease. The results showed that she did.

Beginning the day she was born, in May 2000, at New York Hospital, Perry was examined by Dr. David Abramson, then head of the retinoblastoma team at NY Hospital and now Chief of the OphthalmicOncology Service at Memorial Sloan Kettering. For the first nine months of her life, she received lasertherapy to treat the retinoblastoma tumors, which, fortunately, were small enough not to require enucleation, a procedure that involves surgical removal of the eye.

Following treatment, “She had been tumor-free for months, and things looked really good,” says Larry Zimmerman. “We thought we were well on our way to putting this all behind us.”

Indeed, Perry was healthy and apparently cancer-free between the ages of one and two.

“She was full of energy and happy,” Anne Zimmerman remembers. “We went in for regular check-ups, none of which found anything.”

The Zimmermans knew that less than ten percent of children with inherited retinoblastoma may develop something known as trilateral retinoblastoma, a form of the disease that includes the development of tumors in the brain. “But,” says Anne, “we were under the impression that it was so rare that we weren't expecting Perry to develop it.”

All Hell Breaks Loose

In spring 2002, for more than a week, Perry started exhibiting some strange behaviors. She became extremely lethargic, and her walking gait became bow-legged. Finally, when the behaviors weren't going away and Perry started to develop a frequent headache, the Zimmermans took her to the emergency room. “Even then, we didn't expect it to be anything serious. Maybe just a bad virus,” Anne explains.

They learned that it was more than “just a bad virus,” when, later that night, they received the results of a CT (computed tomography) scan, which showed that Perry had a brain tumor. The frequent headaches were caused by a condition known as hydrocephalus in which the cerebrospinal fluid within the skull can not drain and swelling occurs in the brain. It was 3 AM.

“That was the moment when all hell broke loose,” Larry recalls, shaking his head. “It was the middle of the night. We were told that our daughter had a brain tumor. We didn't know it then, but she was in really bad shape.”

“ That's how you help save your child. You do your research…I believe that, as parents, you do have influence over the outcome, especially in choosing where and how your child will be treated. “

Anne Zimmerman, Perry’s Mother

The very next morning, Dr. Mark M. Souweidane, a pediatricneurosurgeon at New York Hospital, with a joint appointment at Memorial Sloan Kettering, performed surgery to relieve the swelling. “And the next day,” Larry says in astonishment, “Perry was sitting up in bed, singing songs.” The doctors told the Zimmermans that the pressure had most likely been building for at least a month. “When it was finally released,” Larry continues, “it was such a tremendous physical relief. She was happy, and we were crushed because we were still trying to comprehend the news that she had a brain tumor. The gravity of the situation was tremendous. Nobody was giving us any good news. There was nothing for us to hold onto.”

Desperate Decisions

The Zimmerman family

The doctors at New York Hospital told the Zimmermans that Perry would immediately need major surgery to resect, or remove, as much of the tumor as possible. Four days later, Dr. Souweidane performed the eleven-hour surgery. They had been told before the surgery that even with the procedure, Perry had a less than ten percent chance of surviving.

This left them with a critical decision of choosing the best treatment program.

Both Anne and Larry began to seek every bit of information they could find on the latest treatment protocols for trilateral retinoblastoma, making dozens of phone calls and spending countless hours on the Internet. Through their diligent, desperate research, they discovered that, at the time, there were a few patients in the world who had received an intensive treatment protocol that included five chemotherapy cycles followed by a stem cell transplant — which would have to be enough evidence for the Zimmermans.

“That's how you help save your child,” Anne says matter-of-factly. “You do your research. You have to make sure that your child is getting the optimal treatment protocol. You have to understand all the treatments and drugs. Some parents choose not to do this, but I believe that, as parents, you do have influence over the outcome, especially in choosing where and how your child will be treated.”

Once the treatment protocol was chosen, the next decision involved where to send Perry for treatment, a decision which came down to three hospitals: one in Toronto, one in Philadelphia, and Memorial Sloan Kettering. For a number of reasons, including the experience of the staff, the close proximity to their home, and the close connection with Perry's doctors at New York Hospital, they chose Memorial Sloan Kettering.

Treatment Begins

Perry and her brother and sister

Perry started on chemotherapy under the care of Dr. Ira J. Dunkel, a pediatric oncologist at Memorial Sloan Kettering who specializes in brain and eye tumors, and Dr. Yasmin Khakoo, a pediatric neurologist also at the Center. During all of her initial MRI (magnetic resonance imaging) scans, her tumor appeared to be shrinking. Within the first week of Perry's illness, the Zimmermans, in their research, discovered a protocol that included the use of intrathecalmedication — a process in which cancer-fighting drugs are injected directly into the fluid-filled space between the thin layers of tissue that covers the brain. At the time, this was not Memorial Sloan Kettering's standard treatment approach, but the Zimmerman's convinced Dr. Dunkel and Dr. Khakoo to petition the Center's research board to include it in Perry's treatment. Dr. Dunkel received the board's approval, and Perry started receiving intrathecal medication at the start of her fourth chemotherapy cycle.

“Dr. Dunkel and Dr. Khakoo were both so great throughout this entire very trying process,” Anne notes. “We felt that they cared so much for Perry and we knew they would do their best to treat her.”

After the fifth cycle of chemotherapy, before the stem cell transplant, Dr. Souweidane surgically removed any residual tumor. During the seven-hour procedure, the Zimmermans were relieved to learn that the doctors found no active cancer cells remaining.

Larry remembers what a wonderful patient Perry was throughout all of these harrowing procedures. “She was perfect throughout it all. And, in a way, she was the ideal age, where we could spin everything that was happening, saying 'Aren't those nurses great?' and 'Aren't all these tubes and machines fascinating?'”

“We were used to being at the hospital,” Anne adds. “We'd spent so much time there, we were comfortable in that environment. In a strange way, it was more difficult adjusting to going back home.”

Doctors at Memorial rushed her across the street to New York Hospital, where she was placed in a medically induced coma and put on a ventilator for 19 days. The Zimmermans were told by the doctors that they doubted Perry would live through this latest trauma. Both of the Zimmermans remember the experiences of this time as being devastating.

Fortunately, toward the end of the 19 days, Perry's immune system began to recover enough to fight off the virus. It happened to be the week of Christmas, and though they spent it in the hospital, Anne and Larry remember it as their best Christmas ever because Perry became well enough to be taken off the ventilator and removed from the induced coma.

In the wake of her 19 days on a ventilator, Perry could not initially walk or talk due to the stress and muscle deterioration. But within four weeks she had recovered her pre-coma functions, although it took a while for her to get her strength back.

With Cancer Behind Her and a Whole Lifetime Ahead

Perry started pre-school in the fall of 2003 — just eight months after she had finished her treatments at Memorial Sloan Kettering — and is now thriving at Resurrection Episcopal Day School (REDS) in Manhattan. In her new school, she has developed a very special bond with Laurie Boone Hogen, head of REDS.

Ms. Hogen comments: “Perry is a model of courage, compassion, competence, and confidence. After surviving a substantial life and near-death experience at a very young age, Perry has developed an incredible inner strength and has made up her mind to live a full life and realize her considerable gifts. She is an intelligent, joyous child, full of academic potential and an appreciation of life.”

“ Caring for Perry and working with her family has been tremendously rewarding. We are all thrilled that she has done so well despite what seemed to be a very life-threatening diagnosis at the beginning. “

Ira Dunkel, Pediatric Oncologist

Perry continues her check-ups and undergoes diagnostic scanning on a regular basis, and she has had no trace of cancer since completing her treatments in January 2003.

“She's just been great,” Larry says with a deep sense of relieved appreciation.

“I think we're so much better as a family because of this experience,” he continues. “After everything we've been through, we have a completely different outlook on life. We felt like we were pretty grounded before, but an experience like this truly puts life into perspective.”

And Anne's final piece of advice to other parents is clear and succinct: “If there's anything you can do to help ensure that your child is receiving the best treatment that's available, do it.”

If one has any doubt about the outcome of such advice, one need look no farther than bright, adorable, and vivacious Perry Zimmerman.

brain tumor (brayn TOO-mer)

The growth of abnormal cells in the tissues of the brain. Brain tumors can be benign (not cancer) or malignant (cancer).

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

cancer (KAN-ser)

A term for diseases in which abnormal cells divide without control and can invade nearby tissues. Cancer cells can also spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is a cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is a cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is a cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord. Also called malignancy.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

cell (sel)

The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

cerebrospinal fluid (seh-REE-broh-SPY-nul FLOO-id)

The fluid that flows in and around the hollow spaces of the brain and spinal cord, and between two of the meninges (the thin layers of tissue that cover and protect the brain and spinal cord). Cerebrospinal fluid is made by tissue called the choroid plexus in the ventricles (hollow spaces) in the brain. Also called CSF.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

chemotherapy (KEE-moh-THAYR-uh-pee)

Treatment with drugs that kill cancer cells.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

coma (KOH-muh)

A condition in which a patient is in a state of deep sleep and cannot be awakened. A coma may be caused by many things, including trauma, drugs, toxins, or certain diseases.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

condition (kun-DIH-shun)

In medicine, a health problem with certain characteristics or symptoms.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

diagnosis (DY-ug-NOH-sis)

The process of identifying a disease, such as cancer, from its signs and symptoms.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

drain (drayn)

In medicine, to remove fluid as it collects; or, a tube or wick-like device used to remove fluid from a body cavity, wound, or infected area.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

Ensure (en-SHER)

A nutritional drink that may help people who cannot get everything they need in their diet from foods and other drinks. It may be taken by mouth or given through a small tube inserted through the nose into the stomach or the small intestine. It may also be given through a small tube that is put into the stomach or intestinal tract through an opening made on the outside of the abdomen. Ensure is a type of dietary supplement. Also called polymeric enteral nutrition formula.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

enucleation (ee-NOO-klee-AY-shun)

In medicine, the removal of an organ or tumor in such a way that it comes out clean and whole, like a nut from its shell.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

eye cancer (i KAN-ser)

Cancer that forms in tissues of and around the eye. Some of the cancers that may affect the eye include melanoma (a rare cancer that begins in cells that make the pigment melanin in the eye), carcinoma (cancer that begins in tissues that cover structures in the eye), lymphoma (cancer that begins in immune system cells), and retinoblastoma (cancer that begins in the retina and usually occurs in children younger than 5 years).

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

fluid (FLOO-id)

A substance that flows smoothly and takes the shape of its container. Liquids and gases are fluids.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

genetic (jeh-NEH-tik)

Inherited; having to do with information that is passed from parents to offspring through genes in sperm and egg cells.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

high-dose chemotherapy (hy-dose kee-moh-THAYR-uh-pee)

An intensive drug treatment to kill cancer cells, but that also destroys the bone marrow and can cause other severe side effects. High-dose chemotherapy is usually followed by bone marrow or stem cell transplantation to rebuild the bone marrow.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

hydrocephalus (HY-droh-SEH-fuh-lus)

The abnormal buildup of cerebrospinal fluid in the ventricles of the brain.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

immune system (ih-MYOON SIS-tem)

The complex group of organs and cells that defends the body against infections and other diseases.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

inherited (in-HAYR-ih-ted)

Transmitted through genes that have been passed from parents to their offspring (children).

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

intrathecal (IN-truh-THEE-kul)

Describes the fluid-filled space between the thin layers of tissue that cover the brain and spinal cord. Drugs can be injected into the fluid or a sample of the fluid can be removed for testing.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

joint (joynt)

In medicine, the place where two or more bones are connected. Examples include the shoulder, elbow, knee, and jaw.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

laser (LAY-zer)

A device that forms light into intense, narrow beams that may be used to cut or destroy tissue, such as cancer tissue. It may also be used to reduce lymphedema (swelling caused by a buildup of lymph fluid in tissue) after breast cancer surgery. Lasers are used in microsurgery, photodynamic therapy, and many other procedures to diagnose and treat disease.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

M

In chemistry, M is the amount of a substance that has 6.023 x 10(23) atoms or molecules of that substance. Also called mole (chemical).

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

medication (MEH-dih-KAY-shun)

A legal drug that is used to prevent, treat, or relieve symptoms of a disease or abnormal condition.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

MRI

A procedure in which radio waves and a powerful magnet linked to a computer are used to create detailed pictures of areas inside the body. These pictures can show the difference between normal and diseased tissue. MRI makes better images of organs and soft tissue than other scanning techniques, such as computed tomography (CT) or x-ray. MRI is especially useful for imaging the brain, the spine, the soft tissue of joints, and the inside of bones. Also called magnetic resonance imaging, NMRI, and nuclear magnetic resonance imaging.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

mutation (myoo-TAY-shun)

Any change in the DNA of a cell. Mutations may be caused by mistakes during cell division, or they may be caused by exposure to DNA-damaging agents in the environment. Mutations can be harmful, beneficial, or have no effect. If they occur in cells that make eggs or sperm, they can be inherited; if mutations occur in other types of cells, they are not inherited. Certain mutations may lead to cancer or other diseases.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

neurologist (noor-AH-loh-jist)

A doctor who specializes in the diagnosis and treatment of disorders of the nervous system.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

neurosurgeon (NOOR-oh-SER-jun)

A doctor who specializes in surgery on the brain, spine, and other parts of the nervous system.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

oncologist (on-KAH-loh-jist)

A doctor who specializes in treating cancer. Some oncologists specialize in a particular type of cancer treatment. For example, a radiation oncologist specializes in treating cancer with radiation.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

oncology (on-KAH-loh-jee)

The study of cancer.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

ophthalmic (of-THAL-mik)

Having to do with the eye.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

outcome (OWT-kum)

A specific result or effect that can be measured. Examples of outcomes include decreased pain, reduced tumor size, and improvement of disease.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

pediatric (pee-dee-A-trik)

Having to do with children.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

pneumonia (noo-MOH-nyuh)

A severe inflammation of the lungs in which the alveoli (tiny air sacs) are filled with fluid. This may cause a decrease in the amount of oxygen that blood can absorb from air breathed into the lung. Pneumonia is usually caused by infection but may also be caused by radiation therapy, allergy, or irritation of lung tissue by inhaled substances. It may involve part or all of the lungs.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

prenatal (pree-NAY-tul)

Having to do with the time a female is pregnant, before birth occurs. Also called antenatal.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

protocol (PROH-tuh-KOL)

A detailed plan of a scientific or medical experiment, treatment, or procedure. In clinical trials, it states what the study will do, how it will be done, and why it is being done. It explains how many people will be in the study, who is eligible to take part in it, what study drugs or other interventions will be given, what tests will be done and how often, and what information will be collected.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

recover (ree-KUH-ver)

To become well and healthy again.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

regimen (REH-jih-men)

A treatment plan that specifies the dosage, the schedule, and the duration of treatment.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

retinoblastoma (REH-tih-noh-blas-TOH-muh)

Cancer that forms in the tissues of the retina (the light-sensitive layers of nerve tissue at the back of the eye). Retinoblastoma usually occurs in children younger than 5 years. It may be hereditary or nonhereditary (sporadic).

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

scan (skan)

A picture of structures inside the body. Scans often used in diagnosing, staging, and monitoring disease include liver scans, bone scans, and computed tomography (CT) or computerized axial tomography (CAT) scans and magnetic resonance imaging (MRI) scans. In liver scanning and bone scanning, radioactive substances that are injected into the bloodstream collect in these organs. A scanner that detects the radiation is used to create pictures. In CT scanning, an x-ray machine linked to a computer is used to produce detailed pictures of organs inside the body. MRI scans use a large magnet connected to a computer to create pictures of areas inside the body.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

stem cell (stem sel)

A cell from which other types of cells develop. For example, blood cells develop from blood-forming stem cells.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

stem cell transplant (stem sel tranz-plant)

A method of replacing immature blood-forming cells in the bone marrow that have been destroyed by drugs, radiation, or disease. Stem cells are injected into the patient and make healthy blood cells. A stem cell transplant may be autologous (using a patient’s own stem cells that were saved before treatment), allogeneic (using stem cells donated by someone who is not an identical twin), or syngeneic (using stem cells donated by an identical twin).

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

stress (stres)

The response of the body to physical, mental, or emotional pressure. This may make a person feel frustrated, angry, or anxious, and may cause unhealthy chemical changes in the body. Untreated, long-term stress may lead to many types of mental and physical health problems.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

surgery (SER-juh-ree)

A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

therapy (THAYR-uh-pee)

Treatment.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

tissue (TIH-shoo)

A group or layer of cells that work together to perform a specific function.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

trauma (TRAW-muh)

Injury to the body, or an event that causes long-lasting mental or emotional damage.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

tumor (TOO-mer)

An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancer), or malignant (cancer). Also called neoplasm.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

ventilator (VEN-tih-LAY-ter)

In medicine, a machine used to help a patient breathe. Also called respirator.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

virus (VY-rus)

In medicine, a very simple microorganism that infects cells and may cause disease. Because viruses can multiply only inside infected cells, they are not considered to be alive.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)

will (wil)

A legal document in which a person states what is to be done with his or her property after death, who is to carry out the terms of the will, and who is to care for any minor children.

Source: The National Cancer Institute's Dictionary of Cancer Terms(http://www.cancer.gov/dictionary)